Langerhans Cell Granulomatosis Manifested as Pigmented Villonodular Synovitis

Cooley G. Pantazis, MD, Kimberly Templeton, MD, Ossama W. Tawfik, MD, PhD, Raul Braylan, MD

Disclosures

J South Orthop Assoc. 2001;10(4) 

In This Article

Case Report

A 38-year-old white woman had acute onset of right hip pain while jogging approximately 8 months before presentation. The acute pain resolved after protected weight-bearing for 1 week. She complained of a constant, dull ache with any attempt at motion of the hip. On presentation, she was noted to have decreased range of motion of the hip. Radiographs showed permeative lytic lesions in the right femoral head and surrounding right acetabulum (Fig 1). A bone scan (Fig 2) showed increased activity about the right hip. Further evaluation by magnetic resonance imaging (MRI) showed a primarily synovial-based process with extensive invasion into the right femoral head and acetabulum (Fig 3). Results of laboratory studies were normal. The presumptive diagnosis was PVNS. Findings on operative debridement were friable proliferation of synovium, especially the inferior femoral neck, with invasion into the hip capsule, eburnation of the cartilage of the femoral head, and tracking into the acetabular fovea.

Anteroposterior radiograph of pelvis shows lytic lesions in right acetabulum and femoral head (arrows).

Bone scan shows increased uptake around right hip (arrow).

(Top) T1-weighted coronal image of pelvis shows synovial hypertrophy (arrow) with direct invasion into acetabulum and femoral head (arrowheads). (Bottom) Axial T1-weighted image shows direct invasion into acetabulum (arrow).

An intraoperative diagnosis consistent with PVNS was given after frozen section examination of the hip joint tissue. Low-power examination of permanent sections showed the tumor tissue to be composed of cellular polypoid proliferations (Fig 4). The polypoid pannus-like masses were lined by synovial cells and contained histiocytes, eosinophils, hemosiderin, lymphocytes, and multinucleated giant cells (Fig 5). Histologically, the lesions were composed of focal aggregates of Langerhans cells in a polymorphous background of mature eosinophils, lymphocytes, and giant cells. In some microscopic fields, the histiocytes were occurring clusters and nodular collections. At higher magnification, these cells contained distinct cytoplasmic membranes with coffee bean shaped nuclei. Some nuclei contained distinct grooves. On the basis of morphology, Langerhans cell granulomatosis was suspected, and the sections were stained for CD1a and S100. Both markers were positive, confirming the dendritic nature of the cells. Ziehl-Neelsen stain for acid-fast organisms and Gomori's methenamine silver stain failed to show organisms. At this time, the patient was asymptomatic for LCG and was thus followed up. Hip pain improved postoperatively.

Low-power view shows polypoid villous synovial proliferation. (H&E stain, original magnification x 40).

Higher-power view shows heterogeneous population composed of lymphocytes, giant cells, eosinophils, and histocyte-like cells. (H&E stain, original magnification x 400).

Two and one half months later, she presented with abdominal and flank pain, as well as several 0.5 cm cutaneous skin lesions. The abdominal mass and skin lesion were biopsied and removed. The abdominal mass was determined to be enlarged lymph nodes, which histologically were infiltrated by nodular collections of histiocytes (Fig 6). Higher magnification of these histiocytes showed classical Langerhans-type cells (Fig 7). Immunocytochemical markers CD1a and S100 were strongly positive in these cells. The skin pathology revealed a band-like infiltrate involving the dermal and adnexal structures. It was composed primarily of histiocytes with scattered eosinophils and lymphocytes. These histiocyte-like cells were also positive for CD1a and S100 by immunocytochemistry. Computed tomography of the abdomen showed an enlarged mesenteric lymph node (10 cm in diameter). The skin lesion was removed, and the abdominal lymph node was biopsied. Postoperatively, the patient had diabetes insipidus, which responded to desmopressin acetate. Clinically, she continued to show improvement and was eventually treated with vincristine and high-dose prednisone, resulting in resolution of the skin lesions and marked reduction in the abdominal mass.

Abdominal lymph node with nodular collections of histocyte-like cells occurring in sarcoid-like pattern. (H&E stain, original magnification x 100).

Affected lymph nodes with morphologic classical histiocyte-like cell of Langerhans cell granulomatosis. (H&E stain, original magnification x 400).

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